ʻO nā lāʻau hou no ka cystic fibrosis

ʻO Cystic fibrosis (cystic fibrosis) kekahi o nā maʻi lapaʻau maʻamau i nā keiki. No ka hopena o ka anomaly o nā genes, ua hoʻopauʻia ka laweʻana o nā ion ma o nā pēpili pūnaewele, a i ka hopena o ka hoʻohaunaeleʻana i nā hana o nāʻanoʻano he nui. ʻO ka fibrosis pūpū he maʻi noʻonoʻo maʻi autosomal,ʻo ia hoʻi, i mea eʻike pono ai, pono e loaʻa i ke kanakaʻelua kope o nā maʻi kinoʻole, hoʻokahi mai kēlā me kēia makua. ʻO nā mea hou no ka cystic fibrosis e kōkua i kēiaʻano.

Kaʻike o ka cystic fibrosis

ʻO nā hōʻailona a me nā hōʻailona o ka maʻi i maopopo loa, akā e loli ana ma muli o ke koʻikoʻi o ka papa.

Eia kēia mau mea:

■ Ka lawaʻole o ka hana pancreatic (i mālamaʻia i 85% o nā maʻi);

■ ka lawaʻole o ka pulmonary a me ke kūpili (ka hoʻopauʻana o ka bronchial abnormal), e ulu ana ma muli o ka hōʻiliʻiliʻana o ke apo pale ma kaʻaoʻao hanu;

■ Ka hana'onihue liʻiliʻi e pili ana i kaʻoihana ola kinoʻole e alakaʻi ana i ka poho kaumaha a me ka poho kaumaha.

I loko o kaʻohana hoʻokahi, hiki ke loli kaʻinoʻino o ka'ōpū i nā keiki, akā, likeʻole ka maʻiʻeleʻele i ka nui o nā hihia. ʻO ka maʻi pīpī kekahi o nā kumu nui o ka makeʻana o nā maʻi i loaʻa i ka cystic fibrosis. ʻO ka hapa nui pinepine kēia no ka maʻi bacteri i hikiʻole ke mālamaʻia. ʻO ka hoʻonuiʻiaʻana o ka pahu iʻikeʻia ma nā alaloa e hana i nā kūlana maikaʻi no ka uluʻana o nā meaʻano. ʻO nā kānaka e loaʻa ana i ka cystic fibrosis kahi i pili loa i ka maʻi me ka Pseudomonas aeruginosa. Ua hiki i nā pūnaewele maʻi olama ke pale i nā meaʻokoʻa hewaʻole. I nā maʻi me ka cystic fibrosis, ua nele kēia hana, hiki i ka hopena i ka uluʻana o nā maʻi pulmonary.

Hanaʻia o cystic fibrosis

Hoʻonui i nāʻano o ka mālamaʻana i nā cystic fibrosis, pēlā i ka lāʻauʻae a me ka physiotherapy, i mea e hōʻoki i nā akemāmā o ke kīpona, ua hoʻonui i ka emiʻana o ke ola o nā maʻi a hiki i 30 mau makahiki. ʻO ka hapa nui o nā maʻi me ka cystic fibrosis he mea nuiʻole. ʻO ke kumu o ka hānauʻoleʻana o ke kāne,ʻo ka nele loa ia o ka poʻe i lukuʻia,ʻo nā lewa kahi e hele mai ai ka sperm mai nā meaʻokoʻa i ka uruta. I loko o nā wāhine, hiki ke piliʻole ka piliʻole i kaʻike o nā meaʻinoʻole i loko o ka pūpū. Eia nō naʻe, i kēia manawa, e hiki i nā poʻe maʻi ke loaʻa nā keiki me ke kōkua o ka hoʻopunipuni hana. Ma waena o nā mākaʻi o ka lāhui keʻokeʻo keʻokeʻo, hoʻokahi o 25 mau kānaka he mea lawe i ke kāpili cystic fibrosis. Ma muli o ka hoʻomahaʻana o kēia pū, pono e ili mai nā mākua no ka hōʻike o nā hōʻailona maʻi. Ma waena o nā mākaʻi o ka lāhui keʻokeʻo keʻokeʻo,ʻo ka mea halihali o ka pūpū hema o ka cystic fibrosis kahi i 1 kanaka mai 25. A kapaʻia kēlā poʻe heterozygous. ʻAʻole lākou hōʻailona hōʻailona o ka maʻi a me ka pilikia o ka hoʻolālāʻana i ka cystic fibrosis. I loko o ia heluna kanaka, hiki i nā hoaʻelua o ka pahuʻelua ke lawe i ke dia hemahema he 1: 400 (ʻo ia hoʻi, 1 pai o 400). He 50% ka hopena o kēlā me kēia mea lawe i ka hoʻohālikelikeʻana i kēlā me kēia keiki. Ke hoʻoiliʻia nā hoaʻelua i loko o ka pahuʻelua, loaʻa i kēlā me kēia keiki ke kiʻi kūpono o ka pilikia o ka loaʻaʻana o kahi leʻaleʻa hemahema.

■ Ke pilikia o cystic fibrosis ma muli o ka hoʻoilina o nā maʻiʻehaʻelua he 1: 4.

■ Ke pilikia o ka liloʻana i mea halihali i ke kino hemahema i ka wā e loaʻa ai kahi hemahema a hoʻokahi hoʻokahi maʻamau o ka -1: 2.

■ Ke kuleana e loaʻa i nāʻano maʻamau maʻamauʻelua a noho mau i ka helu hemahema-1: 4.

ʻO nā kānaka i hoʻoilina i nā maʻiʻehaʻelua, ua kapaʻia he homozygous, aʻo ka poʻe i hoʻoilina i hoʻokahi pū he heterozygous, a mea lawe paha. Hiki i nā mea halihali ke pilikia i kahi keiki maʻi inā e lawe pū ana kā lāua hoa i kahi lālā hemahema. ʻO nā kānaka pākahiʻole e lawe i ka pahu ke pilikiaʻole i ka hoʻouluʻana i ka maʻi i kā lākou mau keiki e hiki mai ana. ʻO nā wāhine, kahi o kēlā me kēia mea e halihali nei, he 1/2 paha o lākou he keiki maʻi. ʻO ka paʻakikī o ka maʻi ma keʻano likeʻole. Uaʻikeʻia ka hapanui o nā maʻi ma mua o ka makahiki o hoʻokahi makahiki, akā, hiki keʻikeʻia kahiʻanoʻoluʻolu o ka maʻi ma waena o nā makahiki, i kekahi manawa kekahi manawa, i ka wā i nīnauʻia no kaʻikeʻole. ʻO ka nui o ka paʻakai ma kaʻili o kaʻili he mea hōʻailona o cystic fibrosis. ʻO ka "test test" he manawa hou kēia e like me keʻano i hoʻohana muaʻia e nā pale keiki nāna i lapala i ka lae o kahi keiki hānau eʻike i kahi kiʻekiʻe kiʻekiʻe o ka paʻakai i ka wela. ʻOiai uaʻikeʻia he nui ka paʻakai he kiko o ka lawaʻole o ka pulmonary. ʻO Cystic fibrosis kekahi o nā maʻi maʻamau a pau o nā autosomal maʻamau i waena o nā mākaʻi o ka lāhuiʻEleʻele keʻokeʻo aʻoi aʻe ma kaʻawelike i loko o 1 mau 400 mau keiki i hānauʻia. ʻAʻole nā ​​lālā likeʻole he nui ka nui o ka piʻiʻana. No ka laʻana, i nā lālā o ka Hepanic a me ka Latino, he 1 hihia no 9,500 mau keiki hou, a no nā Africa a me nā'Asia, emi mai i ka 1 hihia no 50,000 mau keiki i hānauʻia. Uaʻikeʻia ka hapanui o nā lāhui i noiʻia he emi liʻiliʻi ma mua o nāʻelele o ka lāhuiʻEleʻele keʻokeʻo. Eia nō naʻe, he paʻakikī ke wānana i ke kiʻekiʻe o ka maʻi'āpiopio i loko o kahi heluna lehulehu. Ma kahi o 25% o nā kānaka o Northern Europe e halihali ana i nā maʻi kino o cystic fibrosis. Eia kekahi laʻana, ma UK, loaʻa i kēia maʻi i 1 keiki mai 4,000 hānau (me nā keiki o nā lāhui'ē aʻe, koe wale ke keʻokeʻo).